Pancreatic Islet Cell Tumors

Pancreatic islet cell tumors may be nonfunctioning or functioning tumors. Nonfunctioning tumors may cause obstruction in the shortest part of the small intestine (duodenum) or in the biliary tract, which connects the liver to the duodenum and includes the gall bladder. These nonfunctioning tumors may erode and bleed into the stomach and/or the intestines, or they may cause an abdominal mass. Functioning tumors secrete excessive amounts of hormones, which may lead to various syndromes including low blood sugar (hypoglycemia), multiple bleeding ulcers (Zollinger-Ellison Syndrome), pancreatic cholera (Verner-Morrison Syndrome), carcinoid syndrome or diabetes

Pancreatic islet cell tumors, also known as pancreatic neuroendocrine tumors (PNETs), are rare but potentially serious growths that develop in the pancreas. These tumors can affect the body’s hormonal balance and cause a range of symptoms. In this comprehensive guide, we’ll explain the different types of islet cell tumors, their causes, symptoms, diagnostic tests, treatment options, and available medications in plain and easy-to-understand language.

Types of Pancreatic Islet Cell Tumors:

  1. Insulinomas: These tumors release too much insulin, leading to low blood sugar levels (hypoglycemia).
  2. Glucagonomas: These tumors produce excess glucagon, causing high blood sugar levels (hyperglycemia).
  3. Gastrinomas: Gastrinomas lead to the overproduction of gastrin, which results in increased stomach acid and peptic ulcers.
  4. Somatostatinomas: These tumors produce excessive somatostatin, affecting various hormones in the body.
  5. VIPomas: VIPomas create too much vasoactive intestinal peptide (VIP), causing diarrhea and electrolyte imbalances.
  6. Non-functioning PNETs: These tumors do not produce hormones and may not cause noticeable symptoms until they grow larger.

Causes of Pancreatic Islet Cell Tumors:

  1. Genetic Factors: Some people may inherit genetic mutations that increase their risk of developing these tumors.
  2. Multiple Endocrine Neoplasia Type 1 (MEN1) Syndrome: A rare genetic disorder that predisposes individuals to develop multiple endocrine tumors.
  3. Neurofibromatosis Type 1 (NF1): Another genetic syndrome linked to an increased risk of PNETs.
  4. Tuberous Sclerosis Complex (TSC): This rare genetic condition can also contribute to the development of islet cell tumors.
  5. Sporadic Cases: In many instances, the exact cause of PNETs remains unknown, and they occur sporadically.

Common Symptoms of Pancreatic Islet Cell Tumors:

  1. Hypoglycemia: Abnormally low blood sugar levels can lead to symptoms like confusion, shakiness, and fainting.
  2. Hyperglycemia: High blood sugar levels may cause increased thirst, frequent urination, and fatigue.
  3. Abdominal Pain: Discomfort or pain in the abdomen, often concentrated in the upper part.
  4. Diarrhea: Chronic diarrhea is a common symptom, especially in VIPomas.
  5. Skin Rash: Some individuals with PNETs may develop a skin rash known as necrolytic migratory erythema.
  6. Weight Loss: Unexplained weight loss can occur as a result of the tumor’s effects on the body.
  7. Jaundice: Yellowing of the skin and eyes can occur if the tumor blocks the bile duct.
  8. Peptic Ulcers: Gastrinomas may lead to peptic ulcers, causing abdominal pain and bleeding.
  9. Fatigue: Ongoing fatigue and weakness are common, often due to hormonal imbalances.
  10. Nausea and Vomiting: These symptoms can result from disrupted digestion caused by islet cell tumors.

Diagnostic Tests for Pancreatic Islet Cell Tumors:

  1. Blood Tests: Measuring hormone levels in the blood can help identify overproduction.
  2. Imaging Studies: CT scans, MRI, and ultrasound can visualize the tumor’s location and size.
  3. Endoscopic Ultrasound (EUS): A specialized ultrasound is used to obtain detailed images of the pancreas.
  4. Biopsy: Tissue samples are taken for examination to confirm the tumor’s type and malignancy.
  5. Octreoscan: A nuclear medicine scan that detects neuroendocrine tumors using a radioactive substance.
  6. Chromogranin A Test: Measures the levels of a protein commonly elevated in PNETs.
  7. Genetic Testing: Identifying genetic mutations associated with hereditary forms of PNETs.

Treatment Options for Pancreatic Islet Cell Tumors:

  1. Surgery: Removing the tumor surgically is often the primary treatment if the tumor is localized and operable.
  2. Radiofrequency Ablation (RFA): High-frequency energy is used to destroy tumor cells.
  3. Embolization: Blocking the tumor’s blood supply to shrink it or prepare for surgery.
  4. Chemotherapy: Medications are used to kill cancer cells, especially for advanced or metastatic tumors.
  5. Targeted Therapies: Drugs that specifically target the cancer cells’ unique features.
  6. Somatostatin Analogs: Medications that control hormone secretion in certain types of PNETs.
  7. Peptide Receptor Radionuclide Therapy (PRRT): A radioactive substance is used to target cancer cells.
  8. Liver Transplantation: In some cases, a liver transplant may be considered when the tumor has spread to the liver.

Common Medications for Pancreatic Islet Cell Tumors:

  1. Octreotide (Sandostatin): Helps control hormone secretion in certain types of PNETs.
  2. Lanreotide (Somatuline): Similar to octreotide, it manages hormone-related symptoms.
  3. Everolimus (Afinitor): A targeted therapy that can slow tumor growth in some cases.
  4. Sunitinib (Sutent): Another targeted therapy option for advanced PNETs.
  5. Streptozocin (Zanosar): A chemotherapy drug used in combination with other medications.
  6. Interferon: May be used in some cases to slow tumor growth and control symptoms.
  7. Pasireotide (Signifor): Helps manage symptoms in certain types of islet cell tumors.
  8. Temozolomide (Temodar): Used in combination with other drugs for aggressive PNETs.

Conclusion:

Pancreatic islet cell tumors are uncommon but can have significant effects on the body’s hormonal balance, leading to various symptoms. Identifying the type of tumor, its causes, and appropriate treatment options is crucial for managing this condition effectively. With advancements in medical research and treatment options, individuals diagnosed with pancreatic islet cell tumors have more hope than ever before in managing their condition and improving their quality of life. If you or a loved one are experiencing symptoms suggestive of PNETs, seek medical attention promptly for diagnosis and appropriate care.

 

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. Thank you for giving your valuable time to read the article.

  1. https://medlineplus.gov/skinconditions.html
  2. https://www.aad.org/about/burden-of-skin-disease
  3. https://www.usa.gov/federal-agencies/national-institute-of-arthritis-musculoskeletal-and-skin-diseases
  4. https://www.cdc.gov/niosh/topics/skin/default.html
  5. https://www.skincancer.org/
  6. https://illnesshacker.com/
  7. https://endinglines.com/
  8. https://www.jaad.org/
  9. https://www.psoriasis.org/about-psoriasis/
  10. https://books.google.com/books?
  11. https://www.niams.nih.gov/health-topics/skin-diseases
  12. https://cms.centerwatch.com/directories/1067-fda-approved-drugs/topic/292-skin-infections-disorders
  13. https://www.fda.gov/files/drugs/published/Acute-Bacterial-Skin-and-Skin-Structure-Infections—Developing-Drugs-for-Treatment.pdf
  14. https://dermnetnz.org/topics
  15. https://www.aaaai.org/conditions-treatments/allergies/skin-allergy
  16. https://www.sciencedirect.com/topics/medicine-and-dentistry/occupational-skin-disease
  17. https://aafa.org/allergies/allergy-symptoms/skin-allergies/
  18. https://www.nibib.nih.gov/
  19. https://rxharun.com/rxharun/rxharun/article-types/skin-care-beauty/skin-diseases-types-symptoms-treatment/
  20. https://www.nei.nih.gov/
  21. https://en.wikipedia.org/wiki/List_of_skin_conditions
  22. https://en.wikipedia.org/?title=List_of_skin_diseases&redirect=no
  23. https://en.wikipedia.org/wiki/Skin_condition
  24. https://oxfordtreatment.com/
  25. https://www.nidcd.nih.gov/health/
  26. https://consumer.ftc.gov/articles/w
  27. https://www.nccih.nih.gov/health
  28. https://catalog.ninds.nih.gov/
  29. https://www.aarda.org/diseaselist/
  30. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets
  31. https://www.nibib.nih.gov/
  32. https://www.nia.nih.gov/health/topics
  33. https://www.nichd.nih.gov/
  34. https://www.nimh.nih.gov/health/topics
  35. https://www.nichd.nih.gov/
  36. https://www.niehs.nih.gov
  37. https://www.nimhd.nih.gov/
  38. https://www.nhlbi.nih.gov/health-topics
  39. https://obssr.od.nih.gov/
  40. https://www.nichd.nih.gov/health/topics
  41. https://rarediseases.info.nih.gov/diseases
  42. https://beta.rarediseases.info.nih.gov/diseases
  43. https://orwh.od.nih.gov/

References